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@ARTICLE{RodriguezFos:284773,
author = {E. Rodriguez-Fos and M. Planas-Fèlix$^*$ and M. Burkert
and M. Puiggròs and J. Toedling and N. Thiessen and E.
Blanc and A. Szymansky and F. Hertwig and N. Ishaque and D.
Beule and D. Torrents and A. Eggert and R. P. Koche and R.
F. Schwarz and K. Haase$^*$ and J. H. Schulte and A. G.
Henssen$^*$},
title = {{M}utational topography reflects clinical neuroblastoma
heterogeneity},
journal = {Cell genomics},
volume = {3},
number = {10},
issn = {2666-979X},
address = {Amsterdam},
publisher = {Elsevier},
reportid = {DKFZ-2023-02084},
pages = {100402},
year = {2023},
abstract = {Neuroblastoma is a pediatric solid tumor characterized by
strong clinical heterogeneity. Although
clinicalrisk-defining genomic alterations exist in
neuroblastomas, the mutational processes involved in their
generation remain largely unclear. By examining the
topography and mutational signatures derived from all
variantclasses, we identified co-occurring mutational
footprints, which we termed mutational scenarios. We
demonstrate that clinical neuroblastoma heterogeneity is
associated with differences in the mutational
processesdriving these scenarios, linking risk-defining
pathognomonic variants to distinct molecular
processes.Whereas high-risk MYCN-amplified neuroblastomas
were characterized by signs of replication slippageand
stress, homologous recombination-associated signatures
defined high-risk non-MYCN-amplified patients. Non-high-risk
neuroblastomas were marked by footprints of chromosome
mis-segregation andTOP1 mutational activity. Furthermore,
analysis of subclonal mutations uncovered differential
activity ofthese processes through neuroblastoma evolution.
Thus, clinical heterogeneity of neuroblastoma patientscan be
linked to differences in the mutational processes that are
active in their tumors.},
cin = {BE01},
ddc = {610},
cid = {I:(DE-He78)BE01-20160331},
pnm = {899 - ohne Topic (POF4-899)},
pid = {G:(DE-HGF)POF4-899},
typ = {PUB:(DE-HGF)16},
doi = {10.1016/j.xgen.2023.100402},
url = {https://inrepo02.dkfz.de/record/284773},
}